POS1308 SINGLE CENTER REAL-LIFE PRACTICE OF BIOLOGICAL THERAPY IN SYSTEMIC LUPUS ERYTHEMATOSUS WITH JUVENILE ONSET
Authors: M. Kaleda, I. Nikishina, E. Fedorov, S. Salugina, A. Shapovalenko, T. Pachkoria
Journal: Annals of the Rheumatic Diseases (2022)

Background In recent years the use of Biologics (B) at the management of systemic lupus erythematosus including with juvenile onset (jSLE) has increased. Belimumab (BEL) became the first approved B for SLE. The use of rituximab (RTM), despite the status “of label”, remains relevant in a number of patients (pts) with poor response to standard therapy and the presence of life-threatening conditions. However, use of B in real clinical practice has increase slowly, in part, due to uncertainty over which pts should be treated with B and in what stage of the disease, in part, due to uncertainty over safety. Objectives To analyze in retrospective study the efficacy and safety of B in pts with jSLE. Methods The study included all pts with jSLE in our pediatric rheumatologic department, who received B. Results Among 160 pts with jSLE, 52 (32.5%) received B: 43 – RTM, 12 – BEL, 1 – abatacept (ABA). 4 pts received 2B in sequence (2 – RTM-BEL; 1 - BEL-RTM; 1 – RTM-ABA). The proportion of pts with jSLE was 47% among all those who received RTM in our center. The median age at onset – 12.2 y.o. [IQR 9.9; 14.6], median disease duration at start of RTM – 2.5 years [IQR 0.9; 4.5]. The girls and boys ratio = 6.2:1. The median of activity by SLEDAI at start of RTM was – 16 [12; 22]. 58% of pts underwent more than one course of RTM, with a maximum of 6 courses. The median time between each course was 182 [96–315] days. RTM was effective in 40 (93%) pts with the achievement of the inactive status of the disease and reduction of GC-therapy. Various adverse events (AE) were observed in 46.5% of pts as recurrent uncomplicated upper respiratory tract infections being the most common (23.2%). RTM was discontinued in 9 pts: 1 - the flare of Ro-associated vasculitis, 1 - inefficiency, 7 - serious AEs. Serious AEs included: pneumonia – in 1 (2.3%), serious infusion reaction – 1 (2.3%), toxic epidermolysis – 1 (2.3%), MAS – 2 (4.6%), death (inefficiency for treatment of MAS in SLE) – 2 (4.6%). Among pts receiving BEL the median age at onset was 12.7 y.o. [IQR 10.0; 15.0], median disease duration at start of BEL – 1.5 years [IQR 1.0; 2.1]. The girls and boys ratio = 1.4:1. The median of activity by SLEDAI at start of BEL was – 10 [9; 14]. 6 pts receive BEL more than 1 year with the achievement of the inactive status of the disease and reduction of GC-therapy. AEs were registrated in 3 pts (25%): neutropenia (III degree) – in 1, SAEs – in 2 pts (toxic epidermolysis after 1st infusion, flare of SLE after 6 months of therapy, with discontinuation of BEL in both pts). One patient, boy, 4 y.o. at onset, received ABA (RTM was discontinue after MAS). Disease duration at start of ABA – 7.5 years, the activity by SLEDAI – 10. Treatment with ABA allowed to decrease the activity of jSLE (now SLEDAI=2). Now boy receives ABA during 51 months with good safety, continues GC 5 mg per day, hydroxychloroquine 100 mg per day, MMF 750 mg per day. Conclusion Our study has shown that B is effective in jSLE and has an acceptable safety profile if possible adverse reactions are carefully monitored. In the presence of severe organ lesions, to improve the long-term prognosis, the appointment of B at an early stage of the disease can be discussed. The overall picture that emerges is one of optimism that advances in therapy of jSLE will be realised through the targeted use of an increasing different B. Disclosure of Interests None declared